Transhepatic decompression of the biliary tree in Caroli's disease

Caroli's disease, defined as a congenital cystic dilatation of the intrahepatic segmental bile ducts, has responded poorly to conventional methods of treatment. Medical treatment has not been useful, and the various surgical procedures that have been proposed have met with limited success. The course of this disease is characterized by recurrent episodes of cholangitis, liver failure, and hemorrhage from esophageal varices. Two cases of Caroli's disease are presented. Both failed to respond to standard forms of internal and/or external drainage. However, one of the patients has been asymptomatic for 4 years after insertion of a silicone rubber transhepatic stent, despite suffering from recurrent cholangitis and other symptoms for more than 20 years previously. We therefore propose the use of a silicone rubber transhepatic stent as a possible approach to the treatment problem posed by Caroli's disease.